DR. SHANKAR NARAYAN A

DR. SWASTI PANDEY, DR. JEOTSNA RAJPUT, DR. SHAMA PARVEEN

Abstract

PURPOSE:Goldenhar syndrome is a rare congenital condition characteriszed by cranio-facial anomalies, vertebral and rarely visceral anomalies like cardiovascular defects. It is a unilateral condition. METHODS: A 5 year old boy was brought with a complaint of swelling in both the eyes wit gradual progressive diminution of vision of both eyes since childhood. Parents also complained of defect in the RE upper eyelid. RESULTS:On examination patient had RE upper eyelid coloboma with bilateral limbal dermoid with B/L multiple Auricular tags with bilateral microtia with right hemi-mandibular hypoplasia with cleft palate. His PTA revealed Rt sided Conductive moderate hearing loss and his 2D echo showed situs solitus with Ostium Secundum ASD with left to right shunt with AV-VA concordance. His CXR revealed mild scloliosis to the left side. CONCLUSION:Goldenhar syndrome is a rare congenital anomaly with good prognosis which needs muntidisciplinary treatment from prosthodontist, paediatritian etc.